Portopulmonary hypertension

Clin Liver Dis. 2014 May;18(2):421-38. doi: 10.1016/j.cld.2014.01.004. Epub 2014 Feb 25.

Abstract

Portopulmonary hypertension (POPH) is the presence of pulmonary arterial hypertension in patients with portal hypertension. Among liver transplant (LT) candidates, reported incidence rates of POPH range from 4.5% to 8.5%. In patients with LT, intraoperative death and immediate post-LT mortality are feared clinical events when transplantation is attempted in the setting of untreated, moderate to severe POPH; therefore, POPH precludes LT unless the mean pulmonary artery pressure can be reduced to a safe level and right ventricular function optimized. Specific pulmonary artery vasodilator medications seem effective in reducing pulmonary artery pressures and improving right ventricular function and survival.

Keywords: Cirrhosis; Liver transplant; Portal hypertension; Portopulmonary hypertension; Pulmonary hypertension.

Publication types

  • Review

MeSH terms

  • Cardiac Catheterization
  • Diagnosis, Differential
  • Familial Primary Pulmonary Hypertension / etiology*
  • Familial Primary Pulmonary Hypertension / physiopathology
  • Familial Primary Pulmonary Hypertension / therapy
  • Hemodynamics
  • Humans
  • Hypertension, Portal / complications*
  • Liver Cirrhosis / complications
  • Liver Transplantation
  • Phosphodiesterase 5 Inhibitors / therapeutic use
  • Prostaglandins / therapeutic use
  • Pulmonary Circulation
  • Receptors, Endothelin
  • Ventricular Function, Right

Substances

  • Phosphodiesterase 5 Inhibitors
  • Prostaglandins
  • Receptors, Endothelin